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Special senses: vision and hearing Introduction to the hearing section
Semin Neonatol 2001; 6:499 doi:10.1053/siny.2001.0074, available online at http://www.idealibrary.com on
EDITORIAL
Special senses: vision and hearing Introduction to the hearing section Valerie Newton Permanent hearing loss in infancy is mainly sensorineural with the site of origin being the cochlea. In Western economies the causes of permanent hearing impairment are mainly genetic, whereas infections still play a significant role in developing countries. Recent advances in molecular genetics are now enabling the genetic causes of a hearing impairment to be identified in families and individuals with non-syndromal and syndromal hearing impairment. At present much of the genetic testing is in research establishments but increasingly some tests are becoming available on a service basis. This offers the opportunity of giving parents and affected individuals more informed genetic counselling. As a result, some will choose to have no more children or to limit their families, whereas others will not be deterred by the risks given and others again, where there is a family history of hearing impairment, will be pleased to have the prospect of a hearing impaired child. A permanent hearing impairment in infancy, particularly if it is sensorineural can have major consequences for developing speech and language and for the infant’s emotional, social and educational development. Detection of the hearing impairment as early as possible after birth is now possible as a result of technological advances and this is essential if hearing impaired children are to maximize the opportunity to develop a level of oral communication which is commensurate with that of their normal hearing peers. Habilitation in
1084–2756/02/$-see front matter
the first 6 months of life is necessary to achieve this goal. Should parents prefer for their children to learn to sign then, again, earlier rather than later introduction to signing is in the best interests of the child. Targeted neonatal screening based upon the use of at risk factors has resulted in only about 60% of hearing impaired children being detected as neonates. The problem has been that autosomal recessive non-syndromal hearing loss would not be detected as an at risk factor in a family where no previous hearing impaired child had been born. New autosomal dominant mutations and subclinical intrauterine infections would also be missed in an ‘at risk’ screen. The introduction of universal neonatal screening means that children who were not identifiable as neonates as being at risk for hearing impairment will now be amongst those able to benefit from early detection and early intervention. Early detection programmes cannot be restricted to the neonatal period as some children will acquire a hearing loss later. Service provision must also be in place to identify these, too, as early as possible. In the following articles the causes of a hearing impairment, the methods available for detecting a hearing loss in a neonate and for determining the degree of hearing loss are detailed. The process of habilitation and the use of hearing aid systems to help achieve oral communication are explained. The prospects for infants with a significant hearing impairment are brighter than ever before.
© 2002 Published by Elsevier Science Ltd
EDITORIAL
Special senses: vision and hearing Introduction to the hearing section Valerie Newton Permanent hearing loss in infancy is mainly sensorineural with the site of origin being the cochlea. In Western economies the causes of permanent hearing impairment are mainly genetic, whereas infections still play a significant role in developing countries. Recent advances in molecular genetics are now enabling the genetic causes of a hearing impairment to be identified in families and individuals with non-syndromal and syndromal hearing impairment. At present much of the genetic testing is in research establishments but increasingly some tests are becoming available on a service basis. This offers the opportunity of giving parents and affected individuals more informed genetic counselling. As a result, some will choose to have no more children or to limit their families, whereas others will not be deterred by the risks given and others again, where there is a family history of hearing impairment, will be pleased to have the prospect of a hearing impaired child. A permanent hearing impairment in infancy, particularly if it is sensorineural can have major consequences for developing speech and language and for the infant’s emotional, social and educational development. Detection of the hearing impairment as early as possible after birth is now possible as a result of technological advances and this is essential if hearing impaired children are to maximize the opportunity to develop a level of oral communication which is commensurate with that of their normal hearing peers. Habilitation in
1084–2756/02/$-see front matter
the first 6 months of life is necessary to achieve this goal. Should parents prefer for their children to learn to sign then, again, earlier rather than later introduction to signing is in the best interests of the child. Targeted neonatal screening based upon the use of at risk factors has resulted in only about 60% of hearing impaired children being detected as neonates. The problem has been that autosomal recessive non-syndromal hearing loss would not be detected as an at risk factor in a family where no previous hearing impaired child had been born. New autosomal dominant mutations and subclinical intrauterine infections would also be missed in an ‘at risk’ screen. The introduction of universal neonatal screening means that children who were not identifiable as neonates as being at risk for hearing impairment will now be amongst those able to benefit from early detection and early intervention. Early detection programmes cannot be restricted to the neonatal period as some children will acquire a hearing loss later. Service provision must also be in place to identify these, too, as early as possible. In the following articles the causes of a hearing impairment, the methods available for detecting a hearing loss in a neonate and for determining the degree of hearing loss are detailed. The process of habilitation and the use of hearing aid systems to help achieve oral communication are explained. The prospects for infants with a significant hearing impairment are brighter than ever before.
© 2002 Published by Elsevier Science Ltd